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VASCULITIS SYNDROMES

Emily B. Martin, MD
Rheumatology Board Review
April 9, 2008
KAWASAKI SYNDROME
Mucocutaneous lymph node syndrome
KAWASAKI DISEASE
Diagnostic criteria:
Fever for > 5 days plus 4 of the following:
Bilateral bulbar conjunctival injection
Mucous membrane changes (injected pharynx, cracked lips,
strawberry tongue)
Extremity changes (edema, erythema, or desquamation of
hands or feet)
Polymorphous rash
Cervical lymphadenopathy (at least one >1.5 cm)

CLINICAL MANIFESTIONS
Arthritis and arthralgia
Present in 7-25% of patients
Involves large or small joints
Urethritis
Causes sterile pyuria
CNS involvement
Aseptic meningitis, facial nerve palsy, hearing loss
GI symptoms
Abdominal pain, diarrhea, vomiting, hepatitis
Cardiac involvement
Coronary artery aneurysms, myocardial dysfunction

DIFFERENTIAL DIAGNOSIS
Viral infections
Measles, echovirus, adeno, EBV*
Toxin mediated illnesses
Scarlet fever, toxic shock*
Rickettsial or spirochete infections
Rocky mountain spotted fever*, leptospirosis*
Drug reactions
Stevens-Johnson, serum sickness
JRA
Mercury hypersensitivity reaction
See Mias recent case conference

LABORATORY EVALUATION
Markers of systemic inflammation
Elevated CRP, ESR, leukocytosis with left shift, reactive
thrombocytosis (up to 1 million)
Anemia (normocytic, normochromic)
Sterile pyuria (urethral origin, dont do a cath)
Transaminase elevation (mild to moderate)
CSF findings
Mononuclear pleocytosis, hypoglycorrhachia, elevated
protein
Synovial fluid inflammation
Hyponatremia (increased risk for coronary
aneurysms)


TREATMENT
Mainstay of treatment is IVIG 2 gram/kg over 8-12
hours.
IVIG may need to be repeated in refractory cases.
Several studies have shown that IVIG + aspirin
decreases the risk of coronary aneurysms
compared to aspirin alone.
May also decrease risk of depressed myocardial
function.
High dose aspirin during acute illness then low
dose for about 2 months.
FOR THE BOARDS
Know the clinical manifestations of Kawasaki
syndrome.

Know the differential diagnosis of KD.

Know the laboratory abnormalities seen in KD.

Recognize the value of high-dose IVIG in treatment
of KD.
QUESTION 1
A 3-year-old girl is brought to your office for re-
evaluation of a fever that began 6 days ago. Her
mother tells you that her daughter's temperature
has been as high as 102.2F (39C). Her physical
examination was unremarkable when you
examined her 3 days ago, but today you note
injected sclera; cracked, red lips, a strawberry
appearance of her tongue; and a swollen,
nontender, cervical node. You tell her mother that
you believe this is Kawasaki disease.

QUESTION 1
Of the following, the MOST appropriate statement to make
to the mother is that

A. an exercise stress test should be performed as a
baseline study
B. aspirin therapy will be used until the fever subsides
C. cardiac involvement may include abnormalities of the
coronary arteries or the myocardium
D. echocardiography should be performed to evaluate for
the presence of coronary aneurysms
E. immediate treatment with intravenous immune globulin
will eliminate the chance of coronary involvement

QUESTION 1
Of the following, the MOST appropriate statement to make
to the mother is that

A. an exercise stress test should be performed as a
baseline study
B. aspirin therapy will be used until the fever subsides
C. cardiac involvement may include abnormalities of the
coronary arteries or the myocardium
D. echocardiography should be performed to evaluate for
the presence of coronary aneurysms
E. immediate treatment with intravenous immune globulin
will eliminate the chance of coronary involvement

QUESTION 1
Of the following, the MOST appropriate statement to make
to the mother is that

A. an exercise stress test should be performed as a
baseline study
B. aspirin therapy will be used until the fever subsides
C. cardiac involvement may include abnormalities of the
coronary arteries or the myocardium
D. echocardiography should be performed to evaluate for
the presence of coronary aneurysms
E. immediate treatment with intravenous immune globulin
will eliminate the chance of coronary involvement

HENOCH-SCHONLEIN PURPURA
HENOCH-SCHONLEIN PURPURA
Most common systemic vasculitis in children.
Immune mediated
Deposition of IgA immune complexes.
Often a self-limited disease.
Occurs more often in fall, winter, and spring.
Rare in the summer.
About 50% of cases are preceded by URIs.
Streptococcus is often implicated.
Vaccines, insect bites, viruses have also been reported
as triggers.
CLINICAL PRESENTATION
Classic tetrad
1. Palpable purpura (100%)
In absence of thrombocytopenia or coagulopathy
2. Arthritis or arthralgia (75%)
3. Abdominal pain (50%)
4. Renal disease (21-50%)
GI SYMPTOMS
HSP can cause edema and submucosal
hemorrhage of GI tract.
May see purpuric lesions on endoscopy.
May be the presenting symptom of HSP.
Symptoms typically develop within 8 days of the
rash.
Intussusception is the most common GI
complication.
Be able to recognize obstruction due to HSP.
Know that it is more often ileo-ileal (vs. ileocolic).
RENAL DISEASE
Occurs in up to 50% of patients.
Ranges from hematuria to end-stage renal disease
(<1% of patients).
Usually presents within four weeks of onset of HSP.
Overall prognosis is very good, but there is some
long-term risk of progressive renal impairment.
LABORATORY FINDINGS
There is NO definitive diagnostic test.
IgA levels may be elevated in 50-70% of patients.
Platelet counts and coag studies should be normal.
Inflammatory markers may be elevated.
Urinalysis
Red cells, white cells, casts, proteinuria
May not be present until later in the course
Remember to continue UA screenings after the acute
phase.
Negative RF and ANA.
FOR THE BOARDS
Recognize the typical presentation of HSP.

Recognize that HSP may present initially with
ABDOMINAL PAIN OR JOINT COMPLAINTS.

Recognize INTESTINAL OBSTRUCTION
secondary to HSP.

Know the typical laboratory findings in HSP.
QUESTION 1
A 3-year-old boy is brought to the office with
complaints of intermittent abdominal pain for 2
days. His mother notes that he also had a limp and
a faint rash on his legs for 1 day. He has been
afebrile and otherwise well except for an upper
respiratory tract infection a few weeks ago. On
physical examination, he is alert and complains of
mild abdominal tenderness on palpation. His left
ankle is swollen and tender, and a few 4- to 5-mm
nonblanching lesions (Item Q214A) are visible on
his thighs bilaterally.

QUESTION 1

Of the following, the MOST likely diagnosis is

A. Henoch-Schonlein purpura
B. Immune thrombocytopenic purpura
C. Juvenile rheumatoid arthritis
D. Parvoviral infection
E. Post-streptococcal arthritis
QUESTION 1

Of the following, the MOST likely diagnosis is

A. Henoch-Schonlein purpura
B. Immune thrombocytopenic purpura
C. Juvenile rheumatoid arthritis
D. Parvoviral infection
E. Post-streptococcal arthritis
QUESTION 2
A 3-year-old child presents with a rash, abdominal
pain, and joint pain. Physical exam reveals an
afebrile patient who has a non-blanching
maculopapular rash. The rash is limited to the lower
extremities and buttocks. Both knees are swollen
and tender.

QUESTION 2
Of the following, the laboratory finding that is MOST
consistent with this disease is:

A. Glucosuria
B. Hematuria
C. Positive rheumatoid factor
D. Positive serum antinuclear antibody
E. Thrombocytopenia


QUESTION 2
Of the following, the laboratory finding that is MOST
consistent with this disease is:

A. Glucosuria
B. Hematuria
C. Positive rheumatoid factor
D. Positive serum antinuclear antibody
E. Thrombocytopenia


BEHET DISEASE
BEHET DISEASE
Very rare systemic vasculitis of unknown etiology.
Affects blood vessels of all sizes.
Both arteries and veins
Clinical manifestations are similar in children and
adults.
Characterized by recurrent, painful ulcers of the
mouth and skin and uveitis.

CLINICAL PRESENTATION
Apthous stomatitis
Genital ulcerations
Uveitis
GI symptoms (due to ulcers)
Arthritis
Lab findings
Normal ANA and RF
ESR/CRP may be elevated

SCLERODERMA
Juvenile systemic sclerosis
Localized scleroderma

JUVENILE SYSTEMIC SCLEROSIS
Characterized by symmetrical fibrous thickening of
skin and various internal organs.
Esophagus and GI tract, heart, lungs, kidneys
Clinical presentation
Skin changes edema then tightening, thinning, atrophy
Raynauds 70% at presentation
Arthritis, arthralgia
Muscle weakness/pain
CREST syndrome
Pulmonary fibrosis and pulmonary hypertension are
major causes of morbidity in children.

LOCALIZED SCLERODERMA
Much more common than systemic and has a much
better outcome.
Affects a single dermatome.
Starts as a linear hypopigmented patch then slowly
becomes more fibrotic.
Rarely requires treatment, usually self-limited.
May cause limb deformities or growth arrest.
Lab findings:
RF positive in 1/3 of patients
+/- ANA
FOR THE BOARDS
Recognize the clinical manifestations of
scleroderma.

Recognize that localized scleroderma is much more
common than systemic sclerosis and has a better
outcome.
QUESTION 1
An 11-year-old girl presents with an asymmetric smile
of 6-8 months duration. A tight linear band has
developed progressively from the vermillion border
of the left lower lip and now extends to the lateral
aspect of the chin. Results of the remainder of the
physical exam are normal. No laboratory studies
are obtained.

QUESTION 1
Of the following, the lesion MOST likely represents

A. Granuloma annulare
B. Lichen sclerosis et atrophicus
C. Localized morphea
D. Psoriasis
E. Tuberous sclerosis

Hint: Localized morphea = linear scleroderma
QUESTION 1
Of the following, the lesion MOST likely represents

A. Granuloma annulare
B. Lichen sclerosis et atrophicus
C. Localized morphea
D. Psoriasis
E. Tuberous sclerosis
QUESTION 2
A 14-year-old girl presents for evaluation of areas of
skin thickening, tightness, and discoloration that
developed 2 months ago. Physical examination
reveals shiny, hypopigmented patches with brown
borders on the leg and ankle. The affected skin is
immobile, firm, and has a "bound-down" feeling.

QUESTION 2
Of the following, the MOST likely diagnosis is

A. lichen sclerosus et atrophicus
B. Linear scleroderma
C. Pityriasis alba
D. Progressive systemic sclerosis
E. Vitiligo

QUESTION 2
Of the following, the MOST likely diagnosis is

A. Lichen sclerosus et atrophicus
B. Linear scleroderma
C. Pityriasis alba
D. Progressive systemic sclerosis
E. Vitiligo

OTHER QUESTIONS?